Abstract
Profound congenital sensorineural hearing loss (SNHL) is not so infrequent, affecting 1 to 2 of every 1000 newborns in western countries. Nevertheless, universal hearing screening programs have not been widely applied, although such programs are already established for metabolic diseases. The acquisition of spoken language is a time-dependent process, and some form linguistic input should be present before the first 6 mo of life for a child to become linguistically competent. Therefore, profoundly deaf children should be detected early, and referred timely for the process of auditory rehabilitation to be initiated. Hearing assessment methods should reflect the behavioural audiogram in an accurate manner. Additional disabilities also need to be taken into account. Profound congenital SNHL is managed by a multidisciplinary team. Affected infants should be bilaterally fitted with hearing aids, no later than 3 mo after birth. They should be monitored until the first year of age. If they are not progressing linguistically, cochlear implantation can be considered after thorough preoperative assessment. Prelingually deaf children develop significant speech perception and production abilities, and speech intelligibility over time, following cochlear implantation. Age at intervention and oral communication, are the most important determinants of outcomes. Realistic parental expectations are also essential. Cochlear implant programs deserve the strong support of community members, professional bodies, and political authorities in order to be successful, and maximize the future earnings of pediatric cochlear implantation for human societies.